A case of juvenile advanced liver cirrhosis due to primary sclerosing cholangitis diagnosed on a gastric varices rupture

Abstract

We experienced a case of juvenile advanced liver cirrhosis due to primary sclerosing cholangitis (PSC) diagnosed on a gastric varices rupture. A 23 years old man, suffering from type 2 diabetes treated by pharmacotherapy since 12 years old, visited our hospital due to hematemesis. Gastroduodenoscopy revealed esophago-gastric varices arising from middle of the esophagus to the squamocolumnar junction, and blood clots were observed in the stomach. A red plug existed on the cardiac varices. We treated them by endoscopic sclerotherapy (EIS). Abdominal CT showed an irregular surface of the liver, and dilations of intrahepatic bile ducts in both lobes. Splenomegaly and dilatated umblical vein were also recognized. Endscopic retrograde cholangiopancreatography (ERCP) revealed a beaded appearance of the intrahepatic duct. We performed a liver needle biopsy, and found a pseudolobular fomation with ductular bilirubin stasis as well as an onion-skin type periductal fibrosis around interlobular bile ducts in portal areas. Enentually we diagnosed him as PSC on the basis of these findings. Despite his young age, he developed type 2 diabetes and advanced liver cirrhosis with portal hypertension due to PSC. Hence we should carefully follow him with consideration of liver transplantation as a treatment option.